So is Dwarfism
Dwarfism is a serious statural defect, for which - at the end of puberty - the height does not reach 130 cm in men and 125 cm in women. When the height exceeds these values, but still remains below one meter and a half, we speak of moderate statural hypoevolutism. In any case, given the great variability among the different ethnic groups, the statural reference values remain indicative.
We speak of infantile-dwarfism when in the post-pubertal period the severe somatic deficit is accompanied by the permanence of the characteristic features of childhood. Harmonic dwarfisms are also distinguished from other disharmonious ones; in the first case the body proportions, intellectual and sexual development fall within in the norm, while in the second the natural harmony between the various body segments is lost.
Classification and Causes
He Pingping, with his 73 cm in height, has long been the shortest man in the world (in the photo we see him at the age of 20, shortly before his death on March 13, 2010); his dwarfism was due to a disease known as osteogenesis imperfecta, which includes a heterogeneous group of inherited connective tissue disorders.
All these clarifications are necessary to classify the numerous forms of dwarfism.
One of the most frequent, called pituitary dwarfism, is caused by a defect in the secretion and / or action of GH (somatotropic or growth hormone). This disease causes a severe growth deficit, but there are no defects in intelligence or major anomalies in sexual development (puberty is still delayed). It is therefore a form of harmonic dwarfism. The form of thyroid origin is instead a typical example of disharmonious dwarfism: the limbs are poorly developed with respect to the trunk and the appearance of the face reflects the severe mental deficiency (myxedematous facies, with swollen eyelids and lips, large tongue protruding from the mouth). Sexual development is also severely compromised: secondary sexual characteristics do not mature at puberty and menarche does not appear in women.
Dwarfism can also be of a malformative / disharmonious type; this is the case, for example, of achondroplasia, a hereditary genetic disease, with an autosomal dominant pattern, which affects the cartilages and occurs with a frequency of about one case in 10,000 / 20,000 live births. This incidence makes it the most common among the various forms of dwarfism (responsible for 7 out of 10 cases). The premature welding of the conjugation cartilages is accompanied by the shortness of the limbs, especially of the proximal segments, with respect to the trunk. Thighs and arms are therefore extremely short, while the seated stature is almost normal Intellectual and sexual development are within the norm, the hands are short and square, with the same length as the last four fingers, the head is very voluminous and rounded, with a prominent forehead.
Other forms of dwarfism include: osteogenesis imperfecta (bone dwarfism), mucopolysaccharidosis, Hurler syndrome or gargoilism, Morquio syndrome, Virchow-Secke syndrome and Turner syndrome.
Treatment
For further information: Drugs for the Treatment of Dwarfism
The possibilities of treatment are subordinated to the causes of origin; in children and adolescents affected by pituitary dwarfism, statural growth can be promoted by appropriate hormonal therapies, specifically by daily injections of somatotropin (growth hormone).
The controversial surgical technique of osteogenetic distraction can instead be of help in lengthening, correcting and stabilizing the bone malformations that accompany achondroplastic dwarfism.
